蛋白名称
Glutamate--cysteine ligase catalytic subunit
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Monoclonal, Rabbit,IgG
稀释比例
WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200;
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
其他名称
GCLC ; GLCL ; GLCLC ; Glutamate--cysteine ligase catalytic subunit ; GCS heavy chain ; Gamma-ECS ; Gamma-glutamylcysteine synthetase
背景
Glutamate-cysteine ligase, also known as gamma-glutamylcysteine synthetase is the first rate-limiting enzyme of glutathione synthesis. The enzyme consists of two subunits, a heavy catalytic subunit and a light regulatory subunit. This locus encodes the catalytic subunit, while the regulatory subunit is derived from a different gene located on chromosome 1p22-p21. Mutations at this locus have been associated with hemolytic anemia due to deficiency of gamma-glutamylcysteine synthetase and susceptibility to myocardial infarction.[provided by RefSeq, Oct 2010],
功能
Catalytic activity:ATP + L-glutamate + L-cysteine = ADP + phosphate + gamma-L-glutamyl-L-cysteine.,Disease:Defects in GCLC are the cause of hemolytic anemia [MIM:230450].,enzyme regulation:Feedback inhibition by glutathione.,pathway:Sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine and L-glutamate: step 1/2.,similarity:Belongs to the glutamate--cysteine ligase type 3 family.,subunit:Heterodimer of a catalytic heavy chain and a regulatory light chain.,